摘要目的 探讨先天性岩尖部胆脂瘤的临床特征、诊断及治疗.方法 回顾性分析9例先天性岩尖部胆脂瘤患者的临床表现、影像学检查及手术治疗效果.结果 2例患者早期出现患侧周围性面神经麻痹,进行性加重,4例出现反复性患侧周围性面神经麻痹,均无自觉听力下降；3例先出现听力下降,后出现面神经麻痹.术前9例患者均有不同程度的患侧周围性面神经麻痹和听力下降,7例伴有耳鸣、4例伴有眩晕.术前颞骨高分辨率CT可见岩尖部骨质破坏,MRI检查可见岩尖部占位病变；面神经肌电图及诱发电位检查提示中、重度神经源性改变.8例患者在全麻下行颅中窝进路岩尖部胆脂瘤切除术,1例行颅中窝-迷路联合入路手术.术中见4例面神经水平段裸露、水肿；3例面神经受压变细,行面神经减压术；2例面神经迷路段被病变侵蚀断裂,行端端吻合术.术中彻底切除胆脂瘤病灶,术后随访6～32个月,未见复发.3例患者术后周围性面神经麻痹有不同程度减轻.结论 先天性岩尖部胆脂瘤患者早期可表现为患侧周围性面神经麻痹；术前CT、MRI及面神经功能检查对临床诊断、术式选择及术中面神经处理有重要意义.

abstractsObjective To investigate the clinical symptoms,diagnosis and treatment of congenital petrous apex cholesteatoma.Methods A retrospective review of clinical symptoms,image findings and effect of operation in nine patients with congenital petrous apex cholesteatoma were performed.Results Clinical symptoms:two cases of congenital petrous apex cholesteatoma patients had peripheral facial paralysis,and progressively aggravated; four cases of peripheral facial paralysis were repeated,without hearing loss; three cases firstly appeared hearing loss,followed by paralysis.When hospitalized,nine cases of patients had peripheral facial paralysis and heating loss.Seven patients were complicated with tinnitus,and four had vertigo.HRCT scans showed petrosal apex damage; MRI showed that petrosal apex was occupied;and the facial nerve electromyography and evoked potential examination showed moderate-severe neurogenic changes.All were treated by surgeries,among them,8 cases were by middle fossa approach,1 case was by middle cranial fossa-translabyrinth combined approach.Two cases whose labyrinthine segment of the facial nerve injured severely,were operated by end to end anastomosis; 3 cases with facial nerve compression thinning,were operated by facial nerve decompression; 4 cases were horizontal segment of facial nerve exposed and edema.None of them had recurrence in a follow-up of 6 to 32 months.3 cases of peripheral facial paralysis were lessened in different degrees.Conclusions Patients with congenital petrous apex cholesteatoma have peripheral facial paralysis in the early stage.Computed tomography scanning,magnetic resonance imaging and facial nerve imaging has important significance to clinical diagnosis,choosing the approach of operation and dealing with the facial nerve during operation.